Cet 27 July 2012 Kipioti

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Headaches need not be a headache for optometrists C-19309 O/D

disability. They affect all ages, including children, and there is frequently a positive

family

history.

They

can

be unilateral or bilateral, pulsating, moderate or severe and can last from a few hours to three days. The pain is often localised to the periocular region, or there may be associated visual aura in the form Occasionally, patients report diplopia. Migraine without associated aura often has a strict menstrual relationship. The aura is fully reversible and consists of positive features (eg flickering lights, spots or lines) and/or negative features (eg loss of vision, scotoma). It may be accompanied by fully reversible sensory symptoms, including positive features

(pins

and

needles)

and/or

negative features (numbness) and fully reversible

dysphasic

speech.

Apart

from the visual aura, other premonitory symptoms include photophobia and phonophobia

(aversion

to

noise),

fatigue, neck stiffness, blurred vision and

difficulty

in

concentrating.

Tension-type headache (TTH) With or without peri-cranial tenderness,

Classification

When optometrists are faced with a

In the broad sense, headache is any

patient complaining of headaches, an

pain or ache located in the head, but in

attempt at classifying the disorder as a

practice, only the ones located in the

primary headache (eg migraine, tension

cranial vault are referred to as headaches.

headache) or secondary headache (eg

Headaches have such diverse aetiology

tumour, stroke) should be made. In

that it is has been a significant challenge

general, primary headaches are far

to classify the different types and their

more common and are not related

diagnostic criteria. In 1988, after three

to

years of congresses and combined effort,

whereas secondary headaches are rarer,

the International Headache Society with

but may be a warning sign of a sinister

a headache classification sub-committee

underlying cause. The key to aiding

The

this differentiation is in the history

Cluster headache and trigeminal autonomic cephalalgias (TAC)

International Classification of Headache

and symptoms reported by the patient.

Cluster headache is of particular interest

the

first

edition

of

significant

underlying

pathology,

Disorders with the second, most recent

TTH is the least studied of the primary headache disorders and yet it is, by far, the commonest. Lifetime prevalence in the general population is estimated to be 30-78%4 and is believed to have the highest socio-economic impact. It was previously considered to be primarily psychogenic. bilateral,

The

pressing

pain or

is

typically

tightening

in

quality and of mild to moderate intensity.

to ophthalmologists and optometrists

edition, in 2004.3 In the second edition,

The primary headaches

because of their frequent localisation

45 primary and 120 secondary headache

Migraines

around the eyes. One of the commonest

types and subtypes are identified, as well

These are ranked by the World Health

examples is the ‘short-lasting unilateral

as a further 29 causes of cranial neuralgias

Organization (WHO) as number 19

neuralgiform

headache

attacks

and central causes of facial pain.

among all diseases worldwide causing

conjunctival

injection

and

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tearing

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Tina Kipioti, MD, FRCSEd Of all the painful states that afflict humans, headache (cephalalgia) is the most common. According to a large study,1 95% of all young women and 91% of men experienced headache during a 12-month period and 18% of the women and 15% of the men found their headache significant enough to consult a doctor. More recent figures in the UK corroborate the significance of headache as a problem.2 Patients see an ophthalmologist or optometrist because they experience pain in, or around, the eyes, or other ophthalmic symptoms and signs such as obscuration or visual phenomena. Widespread knowledge of associations between the eyes and headache means that more patients seek an eye specialist’s opinion, so optometrists may examine patients with headaches often before a GP, due to accessibility. This article discusses the most common causes of headaches and offers advice about optometric investigation and diagnosis.

produced

49

of zigzag lines (fortification spectrum).

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(SUNCT)’.

Cluster

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headaches

are

attacks of severe, strictly unilateral

Headache attributed to head and neck trauma

pain, which can be orbital, supraorbital

Headache attributed to cranial or cervical vascular disorder

or temporal, lasting 15-180 minutes

50

and with a typical regular recurrence,

Headache attributed to non-vascular intracranial disorder

from once every other day to eight

Headache attributed to a substance or its withdrawal

times a day. It is often associated with conjunctival

injection,

Headache attributed to infection

lacrimation,

nasal congestion, rhinorrhoea, forehead and facial sweating, miosis, ptosis and eyelid oedema. The patients are typically

Headache attributed to disturbance of homoeostasis Headache or facial pain attributed to disorder of cranium, neck, eyes, ears, nose, sinuses, teeth, mouth or other facial or cranial structures

restless or agitated during an attack (in contrast to the migraine patient, who

Headache attributed to psychiatric disorders

wants to lie down in a quiet room).

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The secondary headaches The

classification

of

secondary

Table 1 Types of secondary headaches

headaches includes those listed in Table 1. Those secondary headaches that are of ocular relevance and consequence are described in the following sections. Giant cell arteritis (GCA) Of the secondary headaches, one of the most important to recognise is GCA, often referred to as temporal arteritis. Pathologically, it is a patchy granulomatous inflammation of medium to large arteries and is not confined to the temporal region. One should always consider GCA if a patient over 50 years of age presents with a headache, especially if it associated with visual symptoms or even visual loss. A blood test (erythrocyte sedimentation rate – ESR and C-reactive protein – CRP) can be diagnostic, although it can also provide a false negative result. Patients often describe their headache as a new type or unusually severe. Other include

classic

symptoms

of

scalp

tenderness,

pain

GCA on

jaw claudication, proximal myalgia, weight loss, malaise, and more rarely, eye or orbital pain (indicating ocular

Figure 1 Papilloedema

ischaemic syndrome). The headache may

fugax), transient diplopia or even cranial

swelling and visual loss is a common

worsen on standing up and be associated

nerve palsies. AION (anterior ischaemic

first presentation of GCA and, again, the

with transient blurred vision (amaurosis

optic neuropathy) with optic nerve

diagnosis of AION in a patient over 50

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years of age with a headache should raise a strong suspicion of temporal arteritis. Acute visual loss in one or both eyes may ensue if not urgently treated with high dose steroids, and it can be fatal. The commonest clinical scenario of GCA is that of an elderly patient with loss of vision in one eye and pain (headache).

51

The temporal arteries may be prominent, examination

the

clinician

confirms

an optic nerve swelling and a visual

Figure 2 Ipsilateral Horner’s Syndrome

field defect, usually altitudinal. Such a

visual obscurations (often postural),

patient needs urgent admission to A&E

photopsias and transient or persistent

and intravenous steroids followed by

diplopia (there may be third, fourth or

systemic steroid treatment for a year.

sixth cranial nerve palsy due to raised ICP). The headache is typically diffuse and constant, aggravated by coughing,

Raised intracranial pressure (ICP) ICP can cause papilloedema. The optic nerve sheaths are an extension of dura around the brain and the sub-arachnoid space

of

the

sheath

contains

CSF

(cerebrospinal fluid), which is in direct communication with the CSF flowing around the brain. When there is high pressure of the CSF, the pressure extends around the optic nerve and results in obstruction of the axoplasmic flow in the optic nerve axons. A build-up of blocked

straining, bending over or lying down and worse in the morning than in the afternoon. Disc swelling is usually bilateral (papilloedema) and necessitates urgent

neuroimaging

(magnetic

resonance imaging – MRI – or magnetic resonance angiogram – MRA) to exclude a space-occupying lesion or venous sinus thrombosis. Monitoring of papilloedema clinically and with Goldmann visual fields and colour vision testing is

with normal consistency of the CSF. Carotid artery dissection Intracranial vascular disorders causing headaches

are

less

common,

but

important to recognise as they are life threatening.

Previous

studies

have

suggested that more than 5% of stroke in young adults is due to dissection (split) of the carotid artery.5 The split in the vessel wall leads to stenosis or complete occlusion of the lumen, resulting in reduced or absent blood flow, which may lead to a cerebrovascular accident (CVA) or stroke. More commonly, clots form on the ragged vessel wall and embolise to the head where they lodge in distal arteries,

essential, as it can result in visual loss.

again resulting in a CVA. Due to the close

becomes visible as a swelling, causing the

Idiopathic intracranial hypertension (IIH)

the sympathetic plexus, 50% of patients

appearance of papilloedema (Figure 1). If

This

to

will get an ipsilateral Horner’s syndrome

pressure is unrelieved, the consequences

as

The

(Figure 2) and reduced blood flow to

are

dysfunction

previous name of benign intracranial

other parts of the brain may result in focal

and eventually death (optic atrophy).

hypertension is now abandoned as it

neurological signs (ie limb weakness on

Raised ICP may be caused by a number

can be very aggressive and refractory to

the opposite side, speech disturbance and

of reasons, the commonest being an

treatment and many patients lose their

visual field loss) if not recognised early.

intracranial space-occupying lesion (eg

vision (complete bilateral blindness

Most cases of carotid artery dissection

a brain tumour or abscess), intracranial

is possible) or have severe disabling

occur spontaneously, although it can

haemorrhage

trauma),

headaches. It is associated with obesity

result from direct head or neck trauma

dural

(except in children, who may have

(eg whiplash) or triggered by a prolonged

venous sinus thrombosis or idiopathic

normal body weight) and patients are

bout of coughing. The accompanying

(pseudotumour cerebri). Symptoms that

usually overweight women, who present

headache is usually gradual in onset

patients may report include blurred

with swollen discs, headaches and often

(occasionally sudden) and deteriorates

vision from optic nerve dysfunction

visual obscurations. Diagnosis is based on

in severity, often accompanied by scalp

or from induced hypermetropia (the

the clinical image, a normal appearance

tenderness and pain in the area of the

eyeball is shortened by pressure from

of the brain on neuroimaging and high

arm and neck. There may be associated

the dilated optic nerve sheath), transient

opening pressure on lumbar puncture

visual

axoplasm in the optic nerve head

optic

nerve

hydrocephalus,

axon

(stroke, meningitis,

is

sometimes

pseudotumour

referred cerebri.

proximity of the internal carotid artery to

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loss

from

ischaemic

optic

27/07/12 CET

inflamed and non-pulsatile, and upon

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neuropathy or retinal artery occlusion and diplopia from cranial nerve palsies.

Headaches

Subarachnoid haemorrhage Symptoms

Examination

Consider

This is a medical emergency and requires an urgent referral to neurology as it is fatal for over 50% of patients within 24 hours

52

Aura

of onset. The great majority of cases are

Chronic headache

Primary headaches (migraine - TTH)

Family history of migraines

due to leakage of blood from an arterial wall defect of the middle cerebral artery, a terminal branch of the internal carotid artery. The blood then spreads between two of the meninges (the membranes that

27/07/12 CET

Daily recurrence

Conjunctival signs

Cluster headaches/ TAC

cover the brain), the pia and arachnoid mater, causing headache and raised ICP. Other causes include venous bleeds,

Patient over 50 years of age

Scalp tenderness

clotting disorders and haemorrhages due Thickened, non-pulsatile temporal arteries

Myalgia Jaw claudication

Malaise

to anticoagulation (warfarin). Typically, it Giant Cell Arteritis

Optic Neuropathy

presents with a ‘thunderclap’ headache, which has an onset within a split second and is frequently described as the ‘worst ever’ that the patient has experienced. Often, it is occipital (back of the head)

Transient

diplopia

Visual Obscurations

in site and may be associated with neck

Worse in the morning

Swollen Optic nerves

Headache change with posture

Enlarged blind spot

stiffness, loss of consciousness, agitation, Raised Intracranial Pressure (ICP)

nausea and vomiting (blood is a very good irritant of the meninges, so it resembles an acute onset of meningitis). Confusion and altered consciousness are poor prognostic indicators, as are focal neurological signs

Deteriorating headache

Diplopia

Visual loss

Arm and Neck pain

Horner’s syndrome

Cranial nerve palsies

(eg limb weakness). Ocular manifestations Carotid Artery Dissection

Focal Neurological Signs

include the features of raised intracranial pressure such as papilloedema and sixth nerve palsies. Infrequently, sub-hyaloid (pre-retinal) haemorrhage with or without vitreous haemorrhage may occur, which

Thunderclap headache Neck stiffness

Nausea & Vomiting Confusion & Altered Consciousness

Papilloedema

6th Nerve Palsy

Subhyaloid Haemorrhage

is referred to as Terson’s syndrome. Subarachnoid Haemorrhage

Dural venous sinus thrombosis Thrombosis of cerebral veins (or venous sinuses) is an uncommon condition

Electric shocklike quality

Unpleasant sensations

Reduced corneal sensation

(although a lot more prevalent than Anisocoria

Trigeminal Neuralgia

previously

thought),

which

often

presents a diagnostic challenge, with a non-specific and, occasionally, dramatic presentation which the optometrist may

Figure 3 Differential diagnosis of headaches

be the first to see. In this condition, one of the cerebral veins (usually the superior sagittal or one of the transverse sinuses)

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becomes obstructed by a clot and ceases

with presbyopia). Confirmation of the

refractive correction)

to drain CSF from the sub-arachnoid

diagnosis is based on the rapid response

• Ocular motility and cover test

space, thus resulting in raised ICP,

to

revealing the presence of heterotropia

headaches and papilloedema, with or

heterophoria or heterotopia may also

and muscle under-actions following

without visual symptoms. The patient

cause recurrent, non-pulsatile, mild to

IIIrd, IVth and VIth nerve palsies

may be otherwise well initially, but as

moderate

usually

• Visual field defects, eg those related

the drainage of cerebral veins remains

absent upon awakening, but worsening

to papilloedema (enlarged blind spot)

obstructed, the slow flow and back-

throughout the day. Headache-inducing

or AION (altitudinal)

pressure may eventually lead to stroke,

heterophoria tends to be either significant

• Anisocoria, and/or fixed dilated

with focal neurological signs, seizures

(close to or at limit of the fusion range)

pupils

and coma. Imaging is paramount for

or intermittent (controlling a large-angle

• Eyelid ptosis (as seen in Horner’s

the diagnosis of this condition, but

divergent

syndrome)

the findings may be subtle and the

include intermittent blurred vision or

• Slit lamp examination of ocular

clinician must have a high index of

diplopia and difficulty adjusting visual

redness and the anterior chamber angle

clinical suspicion to order the correct

focus from distance to near and vice versa.

• Binocular indirect fundoscopy

glasses.

frontal

squint).

Similarly,

headaches,

Other

a

symptoms

(looking for the presence of

the investigation of choice; CT scan alone

Diagnostic approach

will miss a significant number of cases)

When faced with a patient complaining

• Palpate temporal arteries

and

of headaches, one has to remember that the

Figure 3 provides a quick reference

vast majority of headaches are primary or

guide practitioners can use to aid

innocuous, but it is important not to miss

differential diagnosis.

instigate

appropriate

treatment.

Cranial neuralgias, facial pain and other headaches

the few that are caused by a more sinister

53

27/07/12 CET

examination (MRI with venography is

appropriate

papilloedema)

The important cranial neuralgias and

underlying cause. To this effect, the eye

Conclusion

facial

include

care practitioner should pay attention

There

neuritis,

to some important symptoms and signs

course of their career, optometrists

head

that may point to a secondary cause:

are highly likely to be presented with

pains

trigeminal

to

remember

neuralgia,

ophthalmolplegic

optic

‘migraine’,

or facial pain attributed to herpes zoster

and

Tolosa-Hunt

syndrome.

Trigeminal neuralgias may be idiopathic

is

no

doubt

that,

in

the

headache cases, most of which will Symptoms

be benign but others which may be

• History – where, when, triggers of the

life-threatening. Their skill lies in

or secondary due to compression of

headache, any change in the pattern of

identifying these few sinister cases and

the nerve by a tumour or aneurysm, or

pain

making a difference to the patient’s

secondary to multiple sclerosis. It may

• Other neurological symptoms (nausea,

life or vision. In case of uncertainty,

be persistent or recurrent, unilateral

vomiting, tinnitus) or migraineous

a telephone call to emergency eye

or

aura

services for advice may avoid a referral

periocular

and

can

occasionally

have an electric shock-like quality, or unpleasant sensations of ‘pins and

• Headache upon waking or deteriorating with postural changes

needles’ or ‘ants crawling under the

• Neck or arm pain

skin’.

corneal

• Fever or seizures or change in

or facial sensation or the presence of

personality and mental status

anisocoria, increases the risk of a tumour.

• Diplopia, blurred vision or visual

Associated

decreased

Ophthalmic causes of headache include

obscuration

or

indeed

and

expedite

an

appropriate

admission

management.

About the author Tina Kipioti is a consultant ophthalmic surgeon with an interest in paediatrics and strabismus. She trained in the UK,

angle-closure glaucoma, herpes zoster

• Redness or swelling of the eye(s)

Switzerland and Greece. She was clinical

ophthalmicus,

• If the patient is over 50 years of age,

director in ophthalmology, and honorary

uncorrected

refractive

error and heterophoria or heterotropia.

it is important to specifically enquire

Headaches due to refractive error tend to

about other GCA symptoms such as

be recurrent, mild, frontal and/or ocular,

scalp tenderness

are normally absent on awakening and

senior lecturer at Aston University.

References See

www.optometry.co.uk/clinical.

are typically precipitated or aggravated

Signs

Click on the article title and then

by prolonged visual tasks (eg reading

• Reduced visual acuity (with best

on

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‘references’

to

download.

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Module questions Course code: C-19306 O/D (P44-48)

54

1. Which of the following is NOT likely to cause dry eyes? a) Systemic antihistamines b) Increasing age c) Previous laser refractive surgery d) Frequent preservative free lubrication

4. What is the MOST appropriate first line treatment for dry eyes? a) Artificial tear substitutes b) Autologous serum eye drops c) Vitamin A therapy d) Tarsorrhaphy

2. Which of the following signs is NOT associated with dry eyes? a) Congestion of conjunctival vessels b) Filamentary keratitis c) Superficial punctate corneal erosions d) Cells in the anterior chamber

5. Which of the following statements regarding treatment of dry eyes is TRUE? a) Tetracyclines may be effective in treating meibomian gland dysfunction b) Preservative-free medication may exacerbate symptoms of dry eyes c) Dietary modification is not effective for treating dry eyes d) Autologous serum carries no risk as it is derived from the patient’s own blood

27/07/12 CET

3. Which of the following tests may be used in the diagnosis of dry eyes? a) Schirmers Type 1 and 2 b) Tear osmolarity c) Fluorescein and lissamine dye staining d) All of the above

6. Which of the following statements about punctal plugs is TRUE? a) They are used as a last resort in the treatment of dry eyes b) They are only placed in the lower eyelid punctae c)They can cause irritation of the ocular surface if not fitted correctly d) They are a first choice treatment for dry eyes caused by blepharitis

Module questions Course code: C-19309 O/D 1. Which of the following is a common feature of cluster headaches? a) Bilateral eye pain b) Generalised headache c) Diplopia d) Red and watery eye 2. What should you do if a 75-year-old man develops an inferior visual field defect and complains of headaches? a) Enquire about scalp tenderness, jaw pain and loss of weight or malaise b) Perform fixation disparity testing and prescribe the full amount of prism c) Refer him routinely to ophthalmology for further testing (including blood tests) d) Reassure the patient that the headaches are likely to be migraines 3. Which of the following is NOT a common feature of carotid artery dissection? a) Unilateral limb weakness b) Visual field loss c) Colour vision defects d) Horner’s syndrome 4. Which of the following is most likely to be TRUE for a 42-year-old overweight woman who complains of recent onset diplopia and severe head pain?

a) She is likely to have a sixth nerve palsy which warrants correction with prisms b) She is likely to have papilloedema and should be referred as an emergency c)There will be no other signs or symptom associated with this condition d) The underlying condition is likely to be benign and no further action is required 5. Which of the following is TRUE for a 35-year-old man who develops amaurosis fugax and neck pain on the left side, one week after a whiplash injury? a) He is likely to develop sudden onset occipital headaches b) He should be referred routinely to ophthalmology c) There could be a left Horner’s syndrome d) A visual field defect is unlikely to be present 6. Which of the following is MOST consistent with a headache due to refractive error or heterotropia? a) Thunderclap headache, which changes with different posture b) Headache worse in the morning, often waking up the patient c) Unilateral headache or pain around the eye with conjunctival redness and lacrimation d) Mild to moderate chronic / recurrent headache, worse in the evening, relieved by painkillers

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